{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2023,9,16]],"date-time":"2023-09-16T11:10:53Z","timestamp":1694862653364},"reference-count":0,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2006,12,22]],"date-time":"2006-12-22T00:00:00Z","timestamp":1166745600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J of Inher Metab Disea"],"published-print":{"date-parts":[[2007,2]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>We describe a 10\u2010year\u2010old boy with glycogen storage disease type Ib (GSD Ib) with neutropenia and neutrophil dysfunction who never suffered from severe recurrent infections. Lymphocyte subpopulations and assay of intracellular cytokines (IL\u20102, IL\u20104 and IFN\u2010\u03b3) showed a pattern of lymphocyte activation suggesting a shift of T<jats:sub><jats:italic>H<\/jats:italic><\/jats:sub>1\/T<jats:sub><jats:italic>H<\/jats:italic><\/jats:sub>2 balance towards a T<jats:sub><jats:italic>H<\/jats:italic><\/jats:sub>1 response. This is the first report of GSD Ib without severe recurrent infections in spite of neutropenia and neutrophil dysfunction.<\/jats:p>","DOI":"10.1007\/s10545-006-0511-9","type":"journal-article","created":{"date-parts":[[2006,12,22]],"date-time":"2006-12-22T16:32:21Z","timestamp":1166805141000},"page":"105-105","source":"Crossref","is-referenced-by-count":0,"title":["Absence of severe recurrent infections in glycogen storage disease type Ib with neutropenia and neutrophil dysfunction"],"prefix":"10.1002","volume":"30","author":[{"given":"P.","family":"D'Eufemia","sequence":"first","affiliation":[{"name":"Department of Pediatrics University of Rome \u2018La Sapienza\u2019 Rome Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"R.","family":"Finocchiaro","sequence":"additional","affiliation":[{"name":"Department of Pediatrics University of Rome \u2018La Sapienza\u2019 Rome Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M.","family":"Celli","sequence":"additional","affiliation":[{"name":"Department of Pediatrics University of Rome \u2018La Sapienza\u2019 Rome Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"A.","family":"Zambrano","sequence":"additional","affiliation":[{"name":"Department of Pediatrics University of Rome \u2018La Sapienza\u2019 Rome Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M.","family":"Tetti","sequence":"additional","affiliation":[{"name":"Department of Pediatrics University of Rome \u2018La Sapienza\u2019 Rome Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"V.","family":"Ferrucci","sequence":"additional","affiliation":[{"name":"Department of Pediatrics University of Rome \u2018La Sapienza\u2019 Rome Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"L.","family":"Lenti","sequence":"additional","affiliation":[{"name":"Department of Pediatrics University of Rome \u2018La Sapienza\u2019 Rome Italy"},{"name":"Department of Experimental Medicine and Pathology University of Rome \u2018La Sapienza\u2019 Rome Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2006,12,22]]},"container-title":["Journal of Inherited Metabolic Disease"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1007\/s10545-006-0511-9","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1007\/s10545-006-0511-9","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,15]],"date-time":"2023-09-15T04:08:01Z","timestamp":1694750881000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1007\/s10545-006-0511-9"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2006,12,22]]},"references-count":0,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2007,2]]}},"alternative-id":["10.1007\/s10545-006-0511-9"],"URL":"https:\/\/doi.org\/10.1007\/s10545-006-0511-9","archive":["Portico"],"relation":{},"ISSN":["0141-8955","1573-2665"],"issn-type":[{"value":"0141-8955","type":"print"},{"value":"1573-2665","type":"electronic"}],"subject":[],"published":{"date-parts":[[2006,12,22]]}}}